White J G, Gerrard J M
Mol Cell Biochem. 1978 Nov 1;21(2):109-28. doi: 10.1007/BF00240281.
Much of our current knowledge about the physiology of hemostasis has come from intensive study of platelets from patients with inherited and acquired bleeding disorders or an increased risk of thrombotic disease. Appreciation of the role of plasma proteins in platelet stickiness, of platelet surface membrane glyco-proteins in aggregation, of the substances stored in platelet organelles in cell-cell interaction, vascular injury and atherosclerosis, and of endoperoxides and thromboxanes in platelet intercellular communication have resulted largely from investigations on various types of defective platelets. While the techniques of physiology and biochemistry have generated critical details about abnormal platelets, electron microscopy and ultrastructural cytochemistry have provided an improved morphological framework in which to integrate the new discoveries. The present review has attempted to correlate physiological, biochemical and ultrastructural concepts as they relate to the current understanding of inherited platelet disorders.
我们目前关于止血生理学的许多知识都来自对患有遗传性和获得性出血性疾病或血栓形成疾病风险增加的患者的血小板进行的深入研究。对血浆蛋白在血小板黏附、血小板表面膜糖蛋白在聚集、血小板细胞器中储存的物质在细胞间相互作用、血管损伤和动脉粥样硬化以及内过氧化物和血栓素在血小板细胞间通讯中的作用的认识，很大程度上源于对各种类型缺陷血小板的研究。虽然生理学和生物化学技术已经产生了关于异常血小板的关键细节，但电子显微镜和超微结构细胞化学提供了一个改进的形态学框架，以便整合这些新发现。本综述试图将生理学、生物化学和超微结构概念与目前对遗传性血小板疾病的理解联系起来。
